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Leukoencephalopathy after CNS Prophylactic Therapy in Pediatric Hematologic Malignancy (소아 혈액종양 환자에서 중추신경계 예방적 치료 후 발생한 백질뇌병증)

  • Lee, Jun Hwa;Lee, Sun Min;Choi, Eun Jin;Lee, Kun Soo
    • Clinical and Experimental Pediatrics
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    • v.46 no.6
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    • pp.566-571
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    • 2003
  • Purpose : Leukoencephalopathy(LE) is one of the most serious complications in children with hematologic malignancies during the course of treatment. Early recognition is important to reduce the impact and sequelae from LE. We therefore investigated the clinical features of LE following central nervous system(CNS) prophylaxis in children with hematologic malignancies and evaluated the significance of regular check-ups of brain MRI. Methods : We retrospectively reviewed children with hematologic malignancies who had CNS prophylaxis including intrathecal(IT) methotrexate(MTX) and/or cranial irradiation at the Department of Pediatrics, Kyungpook National University Hospital from Oct. 1995 to May 2002. Fifteen cases of acute leukemia and one case of lymphoma who experienced LE following CNS prophylaxis were included in the study. Clinical data were analyzed from the medical records and brain MRIs were reviewed by neuroradiologists. Results : The ages ranged from 1 to 13 years(median age=5.2 years), and the male to female ratio was 3 : 1. The time interval from the beginning of chemotherapy to the time of diagnosis of LE ranged from 2 to 17 months. They all had IT MTX two to 15 times and ten underwent cranial irradiation(1,800 rads). At the time of diagnosis, ten of them had neuropsychiatric symptoms including seizures, personality changes, headache, etc. After the change of treatment modality, four cases showed significant improvement on follow-up MRIs, six cases had no significant changes and two had worsening of LE. Four patients died of infection and bone marrow relapse. Conclusion : CNS prophylaxis with IT therapy and cranial irradiation may cause leukoencephalopathy during the course of treatment. As a result, regular brain MRI check-up is recommended for the early detection and reducing the incidence of LE, along with changes in the treatment modality.

Association of Diagnostic Criteria and Autoantibodies with Juvenile Dermatomyositis in Newly Diagnosed Children (소아기 피부근염의 진단 기준과 자가항체의 진단적 의의)

  • Shin, Kyung Sue;Kim, Joong Gon
    • Clinical and Experimental Pediatrics
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    • v.46 no.9
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    • pp.898-902
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    • 2003
  • Purpose : To determine the clinical association of diagnostic criteria and the prevalence of autoantibodies in newly diagnosed children with juvenile dermatomyositis(JDM). Methods : Thirty-two children with JDM were identified at Seoul National University Children's Hospital from March 1985 to March 1999 by retrospective review. The diagnosis of JDM was based on the criteria proposed by Bohan and Peter. We investigated for the presence of several autoantibodies: antinuclear(ANA), double-stranded DNA, anti-Sm, anti-ribonucleoprotein(RNP), anti-SSA/ SSB, anti-Jo1, anti-Scl-70 antibodies and rheumatoid factor(RF). Results : Sex ratio and age at diagnosis were similar to data published in other studies. All the newly diagnosed children with JDM had a typical rash(100%) and proximal muscle weakness(100%); 17(53%) had muscle pain or tenderness; 10(31%) calcinosis; eight(25%) dysphagia; eight(25%) arthritis, and seven(22%) fever. Muscle enzymes were elevated in 90% of the patients. Of the 27 patients who had an electromyogram, 20(70%) had diagnostic results. Sixteen(70%) of biopsied patients had appropriated results for JDM. Patients were negative for all autoantibodies except ANA and RF. ANA and RF were detected in 47% and 7% of the patients respectively. Conclusion : Although the sensitivity of the criteria proposed by Bohan and Peter is superior, each of these criteria has possible confounding factors. Additional criteria may be needed for early diagnosis of JDM. Based on our findings of autoantibodies in JDM, we do not recommend routine testing for autoantibodies in children with typical JDM.

The Utilities of Lung Biopsy in Pediatric Lung Disease (소아 폐질환에서 폐생검의 유용성)

  • Lee, Jae Hee;Lee, So Yeon;Kim, Ja Hyung;Kim, Bong Sung;Hong, Soo-Jong
    • Clinical and Experimental Pediatrics
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    • v.46 no.12
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    • pp.1230-1234
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    • 2003
  • Purpose : The aim of this study is to evaluate the value of lung biopsies for the management of children with lung disease. Methods : We retrospectively reviewed 19 lung biopsies done at Asan Medical Center, Seoul between 1993 and 2001. Data gathered included demographic information, underlying conditions, diagnosis before biopsy, final diagnosis, change in therapy, morbidity and mortality. Results : Nineteen patients underwent lung biopsy. Among them, 13 patients were male and six patients were female; the median age was 3.6 years(0.8 to 8.6 years). Twelve patients underwent open lung biopsies and seven patients had thoracoscopic biopsies. The overall diagnosis rate was 95 %. The most common diagnosis was interstitial lung disease(12 patients, 64%) and infection was detected in four patients(21%). The biopsy-proven bronchiolitis obliterance was confirmed in two of seven patients suspected by CT findings. Specific treatment was changed after biopsy in 16 patients (85%). The morbidity & overall mortality rates of the patients were 5%(one patient) and 21%(four patients) respectively. Only one complication was seen : empyema. The causes of death were acute respiratory distress syndrome(one patient), respiratory failure(two patients), and septicemia(one patient). Conclusion : The lung biopsy is a safe procedure and it contributes to more accurate diagnosis and proper management of pediatric lung diseases. We recommend lung biopsies should be considered more positively in the diagnosis of pediatric lung diseases.

Relationship between Body Fat Distribution and Metabolic Risk Factors in Adolescents (청소년에서의 체지방 분포와 대사위험 인자들의 연관성)

  • Kim, Dal Hyun;Seong, Tae Jung;Hong, Young Jin;Son, Byong Kwan;Kim, Soon Ki
    • Clinical and Experimental Pediatrics
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    • v.46 no.4
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    • pp.326-334
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    • 2003
  • Purpose : The objectives of this study were to estimate the prevalence of obesity in adolescents; to study the association between obesity, blood pressure, and dyslipidemia; and to evaluate the superiority of waist circumference to waist/hip ratio(WHR) in central obesity. Methods : We selected 1382 adolescents(M : F=690 : 692) aged from 11 to 18 years(mean age; boys 15.0, girls 15.4) in Incheon. We measured weight, height, waist, waist/hip ratio, and blood pressure, with blood sampling to evaluate the dyslipidemia. Results : The prevalence of obesity was 6.7% in boys and 6.9% in girls by body mass index(BMI). The prevalence of systolic hypertension was 15.7% in normal group, 32.4% in the overweight group, and 35.4% in the obese group by BMI. The prevalence of diastolic hypertension was 4.9% in the normal group, 6.9% in the overweight group, and 14.6% the in obese group. In boys and girls, BMI, waist circumference, and WHR were positively correlated with systolic and diastolic blood pressure. respectively. In boys, triglyceride was positively correlated with waist circumference(r=0.34, P<0.05). In boys and girls, BMI was highly correlated with obesity index(r=0.98, each), with waist circumference(r=0.89, r=0.82, respectively), and with body fat(r=0.85, r=0.89) respectively. Conclusion : These data suggest the importance of the screening of blood pressure in obese adolescents, with prevention and intervention of obesity since it may be the most cost-effective way of reducing the complications related with obesity.

Clinical Characteristics and Prognosis of Neonatal Seizures (신생아 경련의 임상적 양상 및 예후에 관한 고찰)

  • Kim, Chang Wu;Jang, Chang Hwan;Kim, Heng Mi;Choe, Byung Ho;Kwon, Soon Hak
    • Clinical and Experimental Pediatrics
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    • v.46 no.12
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    • pp.1253-1259
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    • 2003
  • Backgroud : Seizures in the neonate are relatively common and their clinical features are different from those in children and adults. The study aimed to provide the clinical profiles of neonatal seizure in our hospital. Methods : A total of 41 newborns with seizures were enrolled in this study over a period of three years. They were evaluated with special reference to risk factors, neurologic examinations, laboratory data, neuroimaging studies, EEG findings, seizure types, response to treatment, and prognosis, etc. Results : The average age at onset of seizures was $6.1{\pm}4.6days$ and the majority of patients(42%) had multifocal clonic seizure and 24% had subtle seizure. Factors that are known to increase risk of neonatal seizures include abnormal delivery history, birth asphyxia, and electrolyte imbalance, etc. However, they remain obscure in about 20% of cases. More than 50 percent showed abnormal lesions on neuroimaging studies such as brain hemorrhage, periventricular leukomalacia, brain infarction, cortical dysplasia, hydrocephalus, etc. and 17 out of 32 patients showed abnormal electroencephalographic patterns. Phenobarbital was tried as a first line antiepileptic drug and phenytoin was added if it failed to control seizures. The treatments were terminated in the majority of patients during the hospital stay. The overall prognosis was relatively good except for those with abnormal EEG background or congenital central nervous system malformations. Conclusion : Neonatal seizures may permanently disrupt brain development. Better understanding of their clinical profiles and appropriate management may lead to a reduction in neurological disability in later childhood.

Postoperative Progress and Influencing Factors in Patients after Rastelli Procedure (Rastelli 술식후 임상경과의 평가 및 영향인자)

  • Kim, Se Heui;Kim, Kyeong Sik;Lee, Jong Kyun;Choi, Jae Young;Sul, Jun Hee;Lee, Sung Kyu;Park, Young Whan;Cho, Bum Koo
    • Clinical and Experimental Pediatrics
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    • v.46 no.3
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    • pp.259-264
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    • 2003
  • Purpose : We have performed an analysis on patients who received Rastelli operation in our institute and reviewed their progress postoperatively. Various factors with suspected relationship to the outcome have been considered to help in future treatment and follow-up. Methods : We analyzed retrospectively 43 patients who either received Rastelli operation in Yonsei University Cardiovascular Center from March 1995 to April 1997 or who received post-procedural cardiac catheterization and follow-up echocardiography in the out-patient department after the procedure. Results : No statistically valid relationships were found between the age of the patient, their body weight, preoperative pulmonary arterial index and pressure, presence of pulmonary branchial stenosis and postoperative results. Cases with atrioventricular concordance showed lower age and body weight, and discordant cases exhibited lower ejection fraction 3 days postoperatively. Upon follow up, lower NYHA score was seen in patients with severe residual stenosis. In the group that received cardiac catheterization after the procedure, residual stenosis and right ventricular pressure measurement in echocardiography showed good correlation with the catheterization data. Conclusion : In cases where conduit insertions of the right ventricular outflow tract are required to achieve total correction in complex cardiac deformity, early operation does not seem to provide a clear risk to the patient. In patients with atrioventricular discordance, careful postoperative observation of the ventricular function seems to be needed. Also, echocardiography appears to be a sound method in follow-up of patients after the correctional procedure.

Urinary Tract Infections in Febrile Infants under Three Months of Age (3개월 이하 영아기 열성 요로감염증에 대한 임상적 관찰)

  • Eun, Byung Wook;Chung, Yoo Mi;Kang, Hee Gyung;Ha, Il Soo;Cheong, Hae Il;Lee, Hoan Jong;Choi, Yong
    • Clinical and Experimental Pediatrics
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    • v.46 no.3
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    • pp.265-270
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    • 2003
  • Purpose : To characterize the infants under 3 months of age with urinary tract infections(UTIs), and especially patients with bacteremia or meningitis Methods : Hospital records of all the infants under 3 months of age discharged from our hospital for 69 consecutive months with the diagnosis of initial episode of UTI were reviewed. UTI was defined when patients had fever with pyuria, and had urine culture results of ${\geq}10^5$ colony forming units/mL from a bag specimen. Patients with previously known urologic abnormality or immunodeficiency were excluded. Nosocomial infections were also excluded from the study. Results : The male:female ratio was 35 : 6. Of the urine cultures, 40(97.6%) yielded single pathogen, one yielded two pathogens. Escherichia coli was the predominant isolate from the urine. Five patients(12%) also had bacteremia. Pathogens isolated from the blood cultures were E. coli(4) and Enterococcus faecalis(1). No patient had culture-positive meningitis or cerebrospinal fluid pleocytosis. Clinical or laboratory findings between patients with and without bacteremia were not different significantly. The rate of vesicoureteral reflux(VUR) was 44%. The sensitivity of ultrasound for detection of VUR was 38%; specificity was 50%. Conclusion : Clinical and laboratory data were not helpful for identifying patients with bacteremia at the time of presentation. Consequently, blood cultures need to be obtained from all febrile infants under 3 months of age with UTIs. A large-scale study including the indication of lumbar puncture for infants with a febrile UTI and study of evaluation and treatment of infants under 3 months of age with UTIs are required.

Comparative study of newborns of Asian immigrant and Korean women (외국인과 내국인 산모 출생아의 비교 연구)

  • Park, Hee-ok;Lim, Jae-woo;Jin, Hyun-seung;Shim, Jae-won;Kim, Min-hee;Kim, Chun-soo;Kim, Eun-ryoung;Kim, Seung-youn;Park, Sang-kee;Lee, Jung-joo
    • Clinical and Experimental Pediatrics
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    • v.52 no.10
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    • pp.1119-1126
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    • 2009
  • Purpose:This study compares the maternal characteristics and birth outcomes of infants of Asian immigrant mothers from developing countries with those of the infants of Korean mothers. Methods:In this multicenter and retrospective study, Asian immigrant women who had delivered between January 2005 and June 2008 were enrolled from9 Medical Centers. In all, 333 births to Asian immigrant women from developing countries (Asian-Korean infants) were included in this study. In addition, sex-, birth year-, and gestational age-matched 333 neonates born to Korean mothers were selected as the control group (Korean infants). On the basis of the hospital data, we investigated the nationality, age, and medical history of the mothers and compared the incidence of congenital infection, Apgar score, weight, height, and head circumference of Asian-Korean infants with those of the Korean infants. Results:The average maternal age of Asian women from developing countries at birth term was 26.7 years, which was significantly lower than that of Korean women (30.8 years, P<0.05). The birth weight of Asian-Korean infants (2,869 g) was significantly smaller than that of Korean infants (2,995 g, P<0.05). There was a significant difference in the incidence of congenital syphilis infection between the Asian-Korean infants and Korean infants (5 cases vs. 0 case, P<0.05). Conclusion:There were significant differences in the perinatal outcomes between the Korean and Asian-Korean infants. A multicenter large-scaled study should be performed to analyze the perinatal outcomes of Asian-Korean infants.

The association between serum IGF-1 and neonatal growth and disease in a NICU (신생아집중치료실 입원아의 혈청 IGF-1과 성장 및 질병 사이의 관련성)

  • Kim, Jung Ok;Lim, Hae Ri;Kim, Heng Mi
    • Clinical and Experimental Pediatrics
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    • v.52 no.2
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    • pp.176-180
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    • 2009
  • Purpose : The objective of this study was to establish the serum IGF-1 level in newborn infants, and investigate its association with growth and diseases. Methods : In a retrospective study, serum IGF-1 levels were measured for newborn infants admitted to NICU at Kyungpook University Hospital from March 2007 to July 2007. Birth data, disease history, and hospital course were obtained from medical records. Results : Of 52 blood samples obtained at birth, serum IGF-l levels in 30 preterm infants ($31.6{\pm}27.3$ ng/mL) were lower than in 22 full-term infants ($53.4{\pm}40.0$ ng/mL; P<0.05). In sick full-term infants, serum IGF-1 levels ($46.0{\pm}40.2$ ng/mL) were lower than in healthy full-term infants ($64.1{\pm}39.5$ ng/mL; P<0.05). In preterm infants, there were no differences in IGF-1 levels between healthy ($33.2{\pm}23.3$ ng/mL) and sick infants ($30.6{\pm}30.4$ ng/mL); however, IGF-1 levels in both sick and healthy preterm infants were lower than in healthy full-term infants. Among infants admitted after 8 days of life, serum IGF-1 levels were higher in infants who gained weight ($70.8{\pm}36.2$ ng/mL) than in infants who lost weight ($13.3{\pm}19.9$ ng/mL; P<0.01); however IGF-1 levels showed no difference between gender or method of delivery. Conclusion : The study showed lower IGF-l levels in preterm infants than in full-term infants. Additionally, the IGF-l level in infants with weight loss was lower than in infants with weight gain. These results indicate that serum IGF-1 is associated with gestational age and postnatal growth.

The effectiveness of biochemical indexes for evaluating the nutrition states of children (소아에서 영양평가로서 생화학적 지표의 유용성)

  • Kim, Jae Kwang;Jin, Hyun Seong;Han, Myung Ki;Kim, Bong Seong;Cha, Choong Hwan;Park, Kie Young
    • Clinical and Experimental Pediatrics
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    • v.52 no.2
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    • pp.167-175
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    • 2009
  • Purpose : This study has been conducted to analyze whether the biochemical nutrition indexes might be useful and effective for evaluating the nutrition states of children. Methods : We evaluated 269 children, aged 3-9 years old, who had visited Gangneung Asan Hospital for elective surgery from January 2006 to December 2007, and examined their anthropometric and preoperative laboratory data with retrospective analysis. The children were classified into underweight, normal weight, overweight, and obese groups according to body mass index (BMI). The biochemical nutrition indexes (total lymphocyte count (TLC), hemoglobin, hematocrit, serum albumin, cholesterol, et al) of each group were then analyzed statistically. Results : None of the groups showed statistically significant differences in TLC. Serum albumin decreased significantly in the underweight group. Red blood cell (RBC) count, hemoglobin, hematocrit, and serum total cholesterol in the obese group were higher than in the normal weight group. None of the groups showed statistically significant increase in mean corpuscular volume or mean corpuscular hemoglobin, and it seems that the increase of hemoglobin and RBC count in the overweight and obese groups is due to the enhancement of erythropoiesis rather than iron metabolism. However, in females, almost all nutrition indexes except albumin were statistically significantly poor. Conclusion : Serum albumin, total cholesterol, RBC count, hemoglobin, and hematocrit were useful as nutrition indexes. However, except for albumin, these indexes were significantly poor for females. More control studies are needed to confirm the effectiveness of biochemical indexes for evaluating the nutritional state of children.